Hematology — MCQs

A 5-year-old boy is being evaluated for recurrent epistaxis and other abnormal bleeding episodes, including excessive bleeding from the umbilical cord. Laboratory studies reveal the following: decreased hemoglobin (with microcytic hypochromic red cell indices), normal platelet count, markedly prolonged prothrombin time (PT) and partial thromboplastin time (PTT), and unmeasurable thrombin time (TT). Platelet aggregation studies reveal a normal platelet response to ristocetin, but with other substances (including collagen, ADP, and epinephrine), this patient's platelets exhibit a primary wave defect. Based on these findings, what is the most likely diagnosis?

Q48Medium

Hemolytic crisis in hereditary spherocytosis is most commonly precipitated by which of the following?

Q49Medium

A 9-year-old child presents with continuous bleeding after tonsillectomy. Bleeding time and PTT are prolonged, while platelet count and PT are normal. What is the most likely diagnosis?

Q50Easy

What is the first biochemical change noted after iron therapy is initiated?

Hematology Indian Medical PG Practice Questions and MCQs

Question 41: Which of the following is the most common manifestation of hemophilia?

A. Hemoptysis
B. Hemarthrosis (Correct Answer)
C. Hematemesis
D. Mucosal bleeding

Explanation: **Explanation:** **Hemophilia** (Factor VIII or IX deficiency) is a disorder of secondary hemostasis. Unlike primary hemostatic defects (platelet disorders), which present with superficial bleeding, secondary hemostatic defects typically present with **deep-seated bleeding**. **Why Hemarthrosis is Correct:** Hemarthrosis (bleeding into joint spaces) is the hallmark and most common clinical manifestation of hemophilia, occurring in approximately 75–90% of patients. It most frequently affects the **hinge joints**, with the **knee** being the most common site, followed by the elbow and ankle. Recurrent hemarthrosis leads to "hemophilic arthropathy," characterized by synovial hypertrophy and joint destruction. **Analysis of Incorrect Options:** * **A & C (Hemoptysis and Hematemesis):** These represent internal mucosal or visceral bleeding. While they can occur in severe hemophilia following trauma or underlying pathology (like an ulcer), they are rare and not characteristic primary presentations. * **D (Mucosal bleeding):** This includes epistaxis, gum bleeding, and petechiae. These are classic features of **primary hemostasis defects** (e.g., Von Willebrand Disease or Thrombocytopenia). In hemophilia, mucosal bleeding is uncommon unless there is significant trauma. **High-Yield Clinical Pearls for NEET-PG:** * **Most common joint involved:** Knee > Elbow > Ankle > Hip > Shoulder. * **First sign of hemophilia in infants:** Prolonged bleeding after circumcision or cephalhematoma. * **Muscle Hematomas:** The second most common site of bleeding; the **Iliopsoas muscle** is a high-yield site to remember (can mimic appendicitis). * **Lab Findings:** Prolonged aPTT, normal PT, and normal Bleeding Time (BT). * **Life-threatening complication:** Intracranial hemorrhage is the leading cause of death in hemophiliacs.

Question 42: A 10-year-old boy complains of fatigue and abdominal pain after playing football. He also complains of painful swelling of digits in the past. Ultrasound abdomen shows a small spleen. Which of the following is the most likely diagnosis?

A. Iron deficiency anemia
B. Sickle cell anemia (Correct Answer)
C. Pancreatitis
D. Megaloblastic anemia

Explanation: ### Explanation The clinical presentation is classic for **Sickle Cell Anemia (SCA)**. The key diagnostic clues are: 1. **Dactylitis (Hand-Foot Syndrome):** The "painful swelling of digits" in early childhood is often the first manifestation of SCA, caused by infarcts in the small bones of the hands and feet. 2. **Vaso-occlusive Crisis (VOC):** Fatigue and abdominal pain after physical exertion (football) suggest a crisis triggered by dehydration or hypoxia. Abdominal pain in SCA can result from mesenteric ischemia or splenic sequestration. 3. **Autosplenectomy:** The ultrasound finding of a **"small spleen"** is a pathognomonic late feature of SCA. Repeated splenic infarctions lead to fibrosis and shrinkage of the spleen (autosplenectomy), usually by age 8–10. --- ### Why the other options are incorrect: * **Iron Deficiency Anemia:** While it causes fatigue, it does not present with dactylitis, acute abdominal pain, or splenic atrophy. * **Pancreatitis:** Though it causes abdominal pain, it does not explain the history of painful digits or a shrunken spleen. * **Megaloblastic Anemia:** Presents with fatigue and glossitis, but not with vaso-occlusive phenomena or autosplenectomy. --- ### NEET-PG High-Yield Pearls: * **Splenomegaly in SCA:** The spleen is initially enlarged in early childhood (due to congestion) before undergoing **autosplenectomy**. If a teenager with SCA has a palpable spleen, suspect **HbSC disease** or **S-β thalassemia**. * **Howell-Jolly Bodies:** These nuclear remnants on a peripheral smear indicate functional asplenia/autosplenectomy. * **Most common organism for Osteomyelitis in SCA:** *Salmonella* (though *S. aureus* remains common overall). * **Most common cause of death in adults:** Acute Chest Syndrome. * **Management:** Hydroxyurea is used to increase **HbF** levels, which inhibits sickling.

Question 43: What is the most frequent cause of neonatal thrombocytopenic purpura?

A. Infection (Correct Answer)
B. Drug idiosyncrasy
C. Large haemangiomas
D. Erythroblastosis

Explanation: **Explanation:** **1. Why Infection is the Correct Answer:** In neonatal medicine, **infection (sepsis)** is the most common cause of thrombocytopenia. It occurs in approximately 20–50% of neonates admitted to the NICU. The mechanism is multifactorial: it involves increased platelet consumption due to disseminated intravascular coagulation (DIC), direct bone marrow suppression by toxins, and immune-mediated destruction. Both bacterial (Gram-negative sepsis) and viral (TORCH infections like CMV) etiologies are significant contributors. **2. Analysis of Incorrect Options:** * **Drug Idiosyncrasy (B):** While certain maternal drugs (e.g., thiazides, hydralazine) can cause neonatal thrombocytopenia, this is rare compared to the high incidence of neonatal sepsis. * **Large Haemangiomas (C):** This refers to **Kasabach-Merritt Syndrome**, where platelets are sequestered within a vascular tumor. While it causes severe thrombocytopenia, it is a rare clinical entity. * **Erythroblastosis (D):** In Rh-incompatibility (Erythroblastosis Fetalis), thrombocytopenia can occur due to hypersplenism or marrow replacement by erythroid hyperplasia, but it is far less frequent than infection-induced causes. **3. NEET-PG High-Yield Pearls:** * **Definition:** Neonatal thrombocytopenia is defined as a platelet count **<150,000/µL**. * **Early-onset (<72 hours):** Usually due to placental insufficiency (Preeclampsia, IUGR) or perinatal asphyxia. * **Late-onset (>72 hours):** Almost always due to **Sepsis** or **Necrotizing Enterocolitis (NEC)**. * **Immune causes:** If the neonate is otherwise healthy but has severe thrombocytopenia, consider **Neonatal Alloimmune Thrombocytopenia (NAIT)**—the most common cause of *isolated* severe thrombocytopenia in an otherwise well term infant.

Question 44: A child presents with sudden onset of red spots on the body, preceded by a viral infection 1-4 weeks prior. Investigations show Hb 12 gm%, WBC 11,000, and platelet count of 50,000. Which of the following is not useful for the management of this condition?

A. Platelet transfusion (Correct Answer)
B. Intravenous Immunoglobulin
C. Intravenous anti-D therapy
D. Corticosteroids

Explanation: **Explanation:** The clinical presentation of sudden onset petechiae/purpura following a viral prodrome, with isolated thrombocytopenia (Platelets 50,000/µL) and normal hemoglobin/WBC counts, is classic for **Immune Thrombocytopenic Purpura (ITP)**. **Why Platelet Transfusion is NOT useful (Correct Option):** In ITP, the underlying pathology is the production of autoantibodies (IgG) against platelet surface antigens. These antibody-coated platelets are rapidly destroyed by splenic macrophages. If exogenous platelets are transfused, they are immediately targeted and cleared by the same circulating antibodies. Therefore, platelet transfusion is ineffective and reserved only for life-threatening emergencies (e.g., intracranial hemorrhage). **Why other options are used in management:** * **Corticosteroids (Option D):** The first-line treatment. They decrease antibody production and reduce the clearance of platelets by the reticuloendothelial system. * **Intravenous Immunoglobulin (IVIG) (Option B):** Used for rapid elevation of platelet counts. It works by "clogging" the Fc receptors on splenic macrophages, preventing them from destroying platelets. * **Intravenous anti-D therapy (Option C):** An alternative for Rh-positive patients. It causes mild hemolysis; the spleen becomes busy clearing antibody-coated RBCs, thereby sparing the platelets. **Clinical Pearls for NEET-PG:** * **Bone Marrow Aspiration:** Not mandatory in typical cases but shows **increased megakaryocytes** (compensatory). * **Most common cause of isolated thrombocytopenia** in children. * **Prognosis:** 80% of childhood ITP cases are self-limiting and resolve spontaneously within 6 months. * **Management Threshold:** Treatment is usually initiated if the platelet count is **<20,000/µL** or if there is significant mucosal bleeding.

Question 45: Anemia, splenomegaly, and structural defects are typically seen in which of the following conditions?

A. Thalassemia major (Correct Answer)
B. Thalassemia minor
C. Mediterranean anemia
D. Polycythemia

Explanation: **Explanation:** **Thalassemia Major** (also known as Cooley’s Anemia) is the correct answer because it represents the most severe form of beta-thalassemia, characterized by a near-total lack of beta-globin chain synthesis. This leads to a triad of clinical features: 1. **Severe Anemia:** Ineffective erythropoiesis and hemolysis of defective red blood cells lead to profound microcytic hypochromic anemia, usually manifesting within the first 6–9 months of life. 2. **Splenomegaly:** Massive enlargement occurs due to both extramedullary hematopoiesis and the sequestration of damaged RBCs. 3. **Structural Defects:** To compensate for chronic anemia, the bone marrow undergoes massive expansion. This results in characteristic skeletal changes such as **"Chipmunk facies"** (prominent maxilla, malocclusion of teeth) and a **"Hair-on-end"** appearance on skull X-rays. **Incorrect Options:** * **Thalassemia Minor:** Usually asymptomatic or presents with mild anemia. Patients do not exhibit significant splenomegaly or skeletal deformities. * **Mediterranean Anemia:** This is a historical synonym for Thalassemia. However, in the context of this question, "Thalassemia Major" is the specific clinical entity that defines the severe phenotype described. * **Polycythemia:** This condition involves an *increase* in red blood cell mass, which is the physiological opposite of anemia. **NEET-PG High-Yield Pearls:** * **Target Cells:** Commonly seen on peripheral smears of Thalassemia patients. * **Mentzer Index:** (MCV/RBC count) < 13 suggests Thalassemia trait; > 13 suggests Iron Deficiency Anemia. * **Hb Electrophoresis:** In Thalassemia Major, there is a marked increase in **HbF** and absence/reduction of HbA. * **Iron Overload:** The primary cause of mortality in these patients (due to repeated transfusions), leading to restrictive cardiomyopathy and endocrinopathies.

Question 46: The parents of an 8-year-old boy complained of frequent severe bleeding on minor trauma. The patient could be suffering from which of the following conditions?

A. Hemophilia A (Correct Answer)
B. Anemia
C. Attention Deficit Hyperactive Disorder
D. Disseminated intravascular coagulation

Explanation: **Explanation:** The clinical presentation of frequent, severe bleeding following minor trauma in a young male child is a classic indicator of a **coagulation factor deficiency**, most commonly **Hemophilia A**. **1. Why Hemophilia A is correct:** Hemophilia A is an X-linked recessive disorder caused by a deficiency of **Factor VIII**. It typically manifests in males. The hallmark of the disease is "deep-seated" bleeding. While severe cases present in infancy (e.g., post-circumcision), moderate to mild cases often present in childhood with excessive bruising, hematomas, or **hemarthrosis** (bleeding into joints) following minor physical activity or trauma. **2. Why the other options are incorrect:** * **Anemia:** This refers to a decrease in red blood cells or hemoglobin. While it causes pallor, fatigue, and tachycardia, it does not inherently cause a bleeding diathesis. * **Attention Deficit Hyperactive Disorder (ADHD):** This is a neurobehavioral disorder. While a hyperactive child might sustain more injuries, ADHD itself does not cause a physiological bleeding tendency. * **Disseminated Intravascular Coagulation (DIC):** While DIC causes severe bleeding, it is an **acute, life-threatening consumptive coagulopathy** secondary to systemic illness (sepsis, trauma, malignancy). It would not present as a chronic history of "frequent" bleeding in an otherwise stable 8-year-old. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Hemophilia A and B are X-linked recessive; Hemophilia C (Factor XI deficiency) is Autosomal Recessive. * **Lab Findings:** Characterized by **Prolonged aPTT** with a **Normal PT and Bleeding Time**. * **Mixing Study:** aPTT will **correct** when the patient's plasma is mixed with normal plasma (distinguishes deficiency from inhibitors). * **Treatment:** Recombinant Factor VIII concentrate is the treatment of choice. Desmopressin (DDAVP) can be used in mild cases.

Question 47: A 5-year-old boy is being evaluated for recurrent epistaxis and other abnormal bleeding episodes, including excessive bleeding from the umbilical cord. Laboratory studies reveal the following: decreased hemoglobin (with microcytic hypochromic red cell indices), normal platelet count, markedly prolonged prothrombin time (PT) and partial thromboplastin time (PTT), and unmeasurable thrombin time (TT). Platelet aggregation studies reveal a normal platelet response to ristocetin, but with other substances (including collagen, ADP, and epinephrine), this patient's platelets exhibit a primary wave defect. Based on these findings, what is the most likely diagnosis?

A. Afibrinogenemia (Correct Answer)
B. Bernard-Soulier syndrome
C. Glanzmann's thrombasthenia
D. Wiskott-Aldrich syndrome

Explanation: ### Explanation **Correct Answer: A. Afibrinogenemia** The clinical and laboratory profile points toward a severe deficiency of fibrinogen. 1. **Coagulation Profile:** Fibrinogen is the common final pathway for the coagulation cascade. Its absence leads to an **unmeasurable Thrombin Time (TT)** because TT directly measures the conversion of fibrinogen to fibrin. Consequently, both **PT and PTT are markedly prolonged** as they both rely on fibrin formation as the endpoint. 2. **Platelet Aggregation:** Fibrinogen is essential for platelet aggregation (it binds to the GPIIb/IIIa receptor to form bridges between platelets). In afibrinogenemia, platelets show a **primary wave defect** with ADP, collagen, and epinephrine because they cannot aggregate. However, the response to **ristocetin is normal** because ristocetin-induced agglutination depends on von Willebrand factor and GPIb, not fibrinogen. 3. **Clinical Presentation:** Umbilical cord bleeding is a classic neonatal presentation of afibrinogenemia. --- ### Why the other options are incorrect: * **B. Bernard-Soulier Syndrome:** Characterized by giant platelets and thrombocytopenia. The hallmark is **abnormal ristocetin-induced aggregation** (due to GPIb-IX-V deficiency) that does not correct with normal plasma. PT/PTT/TT are normal. * **C. Glanzmann’s Thrombasthenia:** Caused by a defect in GPIIb/IIIa. While it also shows defective aggregation with ADP/collagen/epinephrine and normal ristocetin response, the **PT, PTT, and TT are always normal** because plasma fibrinogen levels are unaffected. * **D. Wiskott-Aldrich Syndrome:** Presents with the triad of eczema, immunodeficiency, and **microthrombocytopenia** (small platelets). Coagulation studies (PT/PTT/TT) are typically normal. --- ### High-Yield Clinical Pearls for NEET-PG: * **Umbilical Cord Bleeding:** Think of Afibrinogenemia or Factor XIII deficiency. * **Factor XIII Deficiency:** All routine labs (PT, PTT, TT, Platelets) are **normal**. Diagnosis is made via the Urea Clot Solubility test. * **Thrombin Time (TT):** If TT is prolonged, the next step is to check for heparin (corrected by protamine) or fibrinogen deficiency/dysfunction. * **Platelet Aggregation Rule:** * Failure to aggregate with Ristocetin = Bernard-Soulier or vWD. * Failure to aggregate with everything *except* Ristocetin = Glanzmann’s or Afibrinogenemia.

Question 48: Hemolytic crisis in hereditary spherocytosis is most commonly precipitated by which of the following?

A. Parvovirus B19 infection (Correct Answer)
B. Infectious mononucleosis
C. Human T-cell leukemia virus
D. Cytomegalovirus

Explanation: **Explanation:** **Hereditary Spherocytosis (HS)** is a common inherited hemolytic anemia caused by defects in red blood cell (RBC) membrane proteins (like spectrin or ankyrin), leading to spherical, fragile cells that are prematurely destroyed in the spleen. **Why Parvovirus B19 is the correct answer:** While the question uses the term "hemolytic crisis," it specifically refers to the sudden, severe drop in hemoglobin often seen in these patients. **Parvovirus B19** has a unique tropism for erythroid progenitor cells in the bone marrow. In healthy individuals, a temporary halt in RBC production is unnoticed. However, in HS patients—who rely on a high compensatory erythropoietic rate to offset chronic hemolysis—this viral infection causes an **Aplastic Crisis**. This leads to a rapid decline in hemoglobin and a characteristic **low reticulocyte count**, making it the most common and clinically significant crisis in HS. **Why the other options are incorrect:** * **Infectious Mononucleosis (EBV):** While EBV can cause splenomegaly (increasing the risk of splenic rupture in HS), it is not the primary trigger for a hematologic crisis. * **Human T-cell leukemia virus (HTLV):** This is associated with adult T-cell leukemia/lymphoma and tropical spastic paraparesis, not acute crises in hemolytic anemias. * **Cytomegalovirus (CMV):** CMV can cause a mononucleosis-like syndrome but does not specifically target erythroid precursors like Parvovirus B19. **High-Yield Clinical Pearls for NEET-PG:** * **Triad of HS:** Anemia, Jaundice, and Splenomegaly. * **Diagnosis:** Increased MCHC (highly characteristic), positive Osmotic Fragility Test, and the gold standard **EMA Binding Test** (Flow cytometry). * **Aplastic vs. Hemolytic Crisis:** Aplastic crisis (Parvovirus) shows **decreased** reticulocytes; Hemolytic crisis (usually triggered by other infections) shows **increased** reticulocytes. * **Treatment of choice:** Splenectomy (usually deferred until after age 5–6 to reduce the risk of OPSI).

Question 49: A 9-year-old child presents with continuous bleeding after tonsillectomy. Bleeding time and PTT are prolonged, while platelet count and PT are normal. What is the most likely diagnosis?

A. Von Willebrand disease (Correct Answer)
B. Vitamin K deficiency
C. Immune thrombocytopenic purpura
D. Hemophilia A

Explanation: ### Explanation The clinical presentation of post-surgical bleeding combined with a specific laboratory profile points toward **Von Willebrand Disease (vWD)**, the most common inherited bleeding disorder. **Why Von Willebrand Disease is Correct:** Von Willebrand Factor (vWF) has two primary roles: 1. **Platelet Adhesion:** It acts as a bridge between platelets and the subendothelium. A deficiency leads to a defect in the platelet plug formation, resulting in a **prolonged Bleeding Time (BT)**. 2. **Carrier for Factor VIII:** vWF stabilizes Factor VIII in circulation. Low levels of vWF lead to a secondary decrease in Factor VIII, which causes a **prolonged Partial Thromboplastin Time (PTT)**. Since vWD does not affect the extrinsic pathway or platelet production, the **PT and Platelet count remain normal**. **Why Other Options are Incorrect:** * **Vitamin K Deficiency:** Affects Factors II, VII, IX, and X. This would primarily prolong the **PT** (due to Factor VII's short half-life) and PTT, but BT would be normal. * **Immune Thrombocytopenic Purpura (ITP):** Characterized by isolated **thrombocytopenia** (low platelet count). While BT would be prolonged, the PTT would be normal. * **Hemophilia A:** An isolated deficiency of Factor VIII. It causes a prolonged PTT, but the **BT is normal** because platelet-vWF interaction is unaffected. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Most common type (Type 1) is Autosomal Dominant. * **Screening Tests:** Prolonged BT + Prolonged PTT + Normal Platelet Count. * **Confirmatory Test:** Ristocetin Cofactor Assay (measures vWF activity). * **Treatment of Choice:** Desmopressin (DDAVP), which releases stored vWF from Weibel-Palade bodies. For severe cases, use vWF-containing concentrates.

Question 50: What is the first biochemical change noted after iron therapy is initiated?

A. Decreased transferrin saturation (Correct Answer)
B. Reticulocytosis
C. Increase in serum ferritin levels
D. Replenishment of iron stores

Explanation: The sequence of physiological changes following iron therapy is a high-yield topic for NEET-PG. Understanding the timeline of recovery is crucial for monitoring treatment efficacy. ### **Explanation of the Correct Answer** **A. Decreased transferrin saturation:** This is the **earliest biochemical change** (occurring within hours to 24 hours). When therapeutic iron is administered, there is a rapid surge in erythropoiesis. The bone marrow "pulls" iron from the plasma pool to synthesize hemoglobin at an accelerated rate. Because the utilization of iron by the marrow outpaces the rate of intestinal absorption or pharmacological entry into the blood, the serum iron levels and transferrin saturation temporarily drop before they eventually rise. ### **Analysis of Incorrect Options** * **B. Reticulocytosis:** This is the first **hematological** (cellular) response, typically seen within **3–7 days**, peaking at 7–10 days. * **C. Increase in serum ferritin:** Ferritin reflects storage. While it begins to rise as iron is absorbed, it is not the *first* change; it stabilizes much later in the course of therapy. * **D. Replenishment of iron stores:** This is the **last** goal of therapy. It usually takes **3–6 months** of continued treatment after hemoglobin has normalized to fully saturate the body's iron stores. ### **High-Yield Clinical Pearls for NEET-PG** * **First Subjective Sign:** Improvement in well-being and appetite (within 24–48 hours) due to the restoration of iron-containing intracellular enzymes (e.g., cytochromes). * **Hemoglobin Rise:** Hemoglobin typically begins to rise after 1 week, usually at a rate of **0.7–1.0 g/dL per week**. * **Sequence of Recovery Summary:** 1. Intracellular enzyme restoration (Subjective improvement) 2. Decreased transferrin saturation (Biochemical) 3. Reticulocytosis (Hematological) 4. Increase in Hemoglobin 5. Normalization of MCV/MCH 6. Replenishment of stores (Ferritin normalization)

Practice by Chapter

Anemias in Children

Practice Questions

Hemoglobinopathies

Practice Questions

Hemolytic Anemias

Practice Questions

Nutritional Anemias

Practice Questions

Thrombocytopenia

Practice Questions

Bleeding Disorders

Practice Questions

Thrombotic Disorders

Practice Questions

White Blood Cell Disorders

Practice Questions

Bone Marrow Failure Syndromes

Practice Questions

Blood Component Therapy

Practice Questions

Hemophilia and Von Willebrand Disease

Practice Questions

Evaluation of Bleeding Tendencies

Practice Questions

Want unlimited practice?

Get full access to all questions, explanations, and performance tracking.

Start For Free

Hematology — MCQs

Hematology — MCQs

On this page

Practice by Chapter

Want unlimited practice?